Predisposition footprints in the somatic genome of Wilms tumours.

Ten percent of children with cancer harbour a mutation in a predisposition gene. In children with the kidney cancer, Wilms tumour, the prevalence is as high as 30%. Certain predispositions are associated with defined histological and clinical features, suggesting differences in tumourigenesis.

Healthcare providers' expected barriers and facilitators to the implementation of person-centered long-term follow-up care for childhood cancer survivors: A PanCareFollowUp study.

Background: Childhood cancer survivors face high risks of adverse late health effects. Long-term follow-up care for childhood cancer survivors is crucial to improve their health and quality of life. However, implementation remains a challenge.

The purpose of an individual care plan in pediatric palliative care according to healthcare professionals: a qualitative study.

Background: To ensure high-quality pediatric palliative care (PPC) and enable healthcare professionals (HCPs) to provide person-centered care for the individual child with a life-limiting or life-threatening illness and their family, the Individual Care Plan (ICP) for PPC was developed. However, in practice, the ICP for PPC is not used for all children in need of PPC. Insight into why, for which problem and for which children HCPs wish to use an ICP in PPC is therefore necessary to assure that the ICP can be fully implemented in practice.

Parents' and healthcare professionals' experiences with the content of an individual care plan for pediatric palliative care: a mixed-method study.

Background: The Individual Care Plan (ICP) for pediatric palliative care translates the general guideline recommendations into a personalized plan for the child. Various documents exist in pediatrics globally, aimed at facilitating anticipatory care or coordinating end-of-life care. The ICP aims both, but user experiences have not been studied post-development.

A practical guide to therapeutic drug monitoring in busulfan: recommendations from the Pharmacist Committee of the European Society for Blood and Marrow Transplantation (EBMT).

Busulfan (Bu) is an important component of many conditioning regimens for allogeneic hematopoietic cell transplantation. The therapeutic window of Bu is well characterized, with strong associations between Bu exposure and the clinical outcome in adults (strongest evidence in myelo-ablative setting) and children (all settings). We provide an overview of the literature on Bu as well as a step-by-step guide to the implementation of Bu therapeutic drug monitoring (TDM).

The Practical Application of the Individual Care Plan for Pediatric Palliative Care: A Mixed-Method Study.

Background/objective: The Individual Care Plan (ICP) for pediatric palliative care was developed to provide person-centered care for the individual child and family. Currently, a lack of clarity remains regarding the use and function of the ICP in daily practice. To further implement the ICP, it is important to identify how parents and healthcare professionals use the ICP and which obstacles or benefits are experienced.

Evaluating the Clinical Impact and Feasibility of Therapeutic Drug Monitoring of Pazopanib in a Real-World Soft-Tissue Sarcoma Cohort.

Introduction And Objective: Pazopanib is registered for metastatic renal cell carcinoma and soft-tissue sarcoma (STS). Its variable pharmacokinetic (PK) characteristics and narrow therapeutic range provide a strong rationale for therapeutic drug monitoring (TDM). Prior studies have defined target levels of drug exposure (≥ 20.

Adherence to low-dose methotrexate in children with juvenile idiopathic arthritis using a sensitive methotrexate assay.

Background: Low-dose weekly methotrexate (MTX) is the mainstay of treatment in juvenile idiopathic arthritis. Unfortunately, a substantial part of patients has insufficient efficacy of MTX. A potential cause of this inadequate response is suboptimal drug adherence.

Automated radiolabelling of [Ga]Ga-PSMA-11 (gallium (Ga)-gozetotide) using the Locametz® kit and two generators.

Background: Steps have been taken by pharmaceutical companies to obtain marketing authorisation of PSMA ligands in the European Union. Since December 2022, Locametz® (PSMA-11, gozetotide) is licensed as kit for manual radiolabelling with gallium-68 and commercially available since mid-2023. The Summary of Product Characteristic (SmPC) describes manual radiolabelling with a maximum activity after radiolabelling of 1369 MBq.

Improving advanced intraoperative MRI methods during pediatric neurosurgery.

Advanced intraoperative MR images (ioMRI) acquired during the resection of pediatric brain tumors could offer additional physiological information to preserve healthy tissue. With this work, we aimed to develop a protocol for ioMRI with increased sensitivity for arterial spin labeling (ASL) and diffusion MRI (dMRI), optimized for patient positioning regularly used in the pediatric neurosurgery setting. For ethical reasons, ASL images were acquired in healthy adult subjects that were imaged in the prone and supine position.

The applicability of the central line-associated bloodstream infection (CLABSI) criteria for the evaluation of bacteremia episodes in pediatric oncology patients.

Background: The aim of this study was to investigate the applicability of the central line-associated bloodstream infection (CLABSI) criteria of the Centers for Disease Control and Prevention in pediatric oncology patients.

Methods: Bacteremia episodes from 2020 to 2022 from a prospective cohort of pediatric oncology patients with a central venous catheter were included. Episodes were classified by three medical experts following the CLABSI criteria as either a CLABSI or non-CLABSI (i.

Shared decision-making in pediatric palliative care in the Netherlands.

Shared decision-making (SDM) is a process in which health care professionals (HCPs) involve parents and children - when appropriate- to decide together on future treatment. These decisions are based on values that are important for the family, goals of care and preferences for future care and treatment. Elucidation of these values and preferences is preferably done early in the disease trajectory via so-called Advance Care Planning (ACP) conversations.

Palliative care for children: methodology for the development of a national clinical practice guideline.

Background: Provision of paediatric palliative care for children with life-threatening or life-limiting conditions and their families is often complex. Guidelines can support professionals to deliver high quality care. Stakeholders expressed the need to update the first Dutch paediatric palliative care guideline with new scientific literature and new topics.

Central Venous Catheter-related Bloodstream Infections Caused by Enterobacterales in Pediatric Oncology Patients: Catheter Salvage or Removal.

Background: The aim was to determine whether salvage treatment with systemic antibiotics is a safe and effective strategy for Enterobacterales bloodstream infections (BSI) in pediatric oncology patients with a central venous catheter (CVC).

Methods: A retrospective study was performed on oncology and stem cell recipient patients with a CVC and blood culture with Enterobacterales , at the Princess Máxima Centre for Pediatric Oncology, Utrecht, the Netherlands. Analyses were performed for all BSI and for episodes meeting central line-associated bloodstream infection (CLABSI) criteria.

Distinct clinical phenotypes in paediatric cancer patients with sepsis are associated with different outcomes-an international multicentre retrospective study.

Background: Identifying phenotypes in sepsis patients may enable precision medicine approaches. However, the generalisability of these phenotypes to specific patient populations is unclear. Given that paediatric cancer patients with sepsis have different host response and pathogen profiles and higher mortality rates when compared to non-cancer patients, we determined whether unique, reproducible, and clinically-relevant sepsis phenotypes exist in this specific patient population.

Treatment outcomes of childhood PICALM::MLLT10 acute leukaemias.

The prognostic impact of PICALM::MLLT10 status in childhood leukaemia is not well described. Ten International Berlin Frankfurt Münster-affiliated study groups and the Children's Oncology Group collaborated in this multicentre retrospective study. The presence of the PICALM::MLLT10 fusion gene was confirmed by fluorescence in situ hybridization and/or RNA sequencing at participating sites.

regulates one-carbon metabolism and histone methylation in ground-state pluripotency.

Understanding mechanisms of epigenetic regulation in embryonic stem cells (ESCs) is of fundamental importance for stem cell and developmental biology. Here, we identify , a member of the ETS family of transcription factors (TFs), as a marker of ground state pluripotency. We show that is rapidly induced in ground state ESCs and in response to extracellular signal-regulated kinase (ERK) inhibition.

Maintenance Chemotherapy for Patients with Rhabdomyosarcoma.

Maintenance chemotherapy (MC) defines the administration of prolonged relatively low-intensity chemotherapy with the aim of "maintaining" tumor complete remission. This paper aims to report an update of the RMS2005 trial, which demonstrated better survival for patients with high-risk localized rhabdomyosarcoma (RMS) when MC with vinorelbine and low-dose cyclophosphamide was added to standard chemotherapy, and to discuss the published experience on MC in RMS. In the RMS2005 study, the outcome for patients receiving MC vs.

Advanced intraoperative MRI in pediatric brain tumor surgery.

In the pediatric brain tumor surgery setting, intraoperative MRI (ioMRI) provides "real-time" imaging, allowing for evaluation of the extent of resection and detection of complications. The use of advanced MRI sequences could potentially provide additional physiological information that may aid in the preservation of healthy brain regions. This review aims to determine the added value of advanced imaging in ioMRI for pediatric brain tumor surgery compared to conventional imaging.

Machine Learning Logistic Regression Model for Early Decision Making in Referral of Children with Cervical Lymphadenopathy Suspected of Lymphoma.

While cervical lymphadenopathy is common in children, a decision model for detecting high-grade lymphoma is lacking. Previously reported individual lymphoma-predicting factors and multivariate models were not sufficiently discriminative for clinical application. To develop a diagnostic scoring tool, we collected data from all children with cervical lymphadenopathy referred to our national pediatric oncology center within 30 months ( = 182).

Improvement and implementation of a national individual care plan in paediatric palliative care: a study protocol.

Introduction: Paediatric palliative care (PPC) is care for children with life-threatening or life-limiting conditions, and can involve complex high-tech care, which can last for months or years. In 2015, the National Individual Care Plan (ICP) for PPC was developed and has shown to be successful. The ICP can be seen as an instrument to facilitate coordination, quality and continuity of PPC.

Exposure of anti-infective drugs and the dynamic changes of the gut microbiota during gastrointestinal mucositis in autologous stem cell transplant patients: a pilot study.

Gastrointestinal mucositis could potentially compromise drug absorption due to functional loss of mucosa and other pathophysiological changes in the gastrointestinal microenvironment. Little is known about this effect on commonly used anti-infectives. This study aimed to explore the association between different stages of gastrointestinal mucositis, drug exposure, and gut microbiota.

Agonist-controlled competition of RAR and VDR nuclear receptors for heterodimerization with RXR is manifested in their DNA binding.

We found previously that nuclear receptors (NRs) compete for heterodimerization with their common partner, retinoid X receptor (RXR), in a ligand-dependent manner. To investigate potential competition in their DNA binding, we monitored the mobility of retinoic acid receptor (RAR) and vitamin D receptor (VDR) in live cells by fluorescence correlation spectroscopy. First, specific agonist treatment and RXR coexpression additively increased RAR DNA binding, while both agonist and RXR were required for increased VDR DNA binding, indicating weaker DNA binding of the VDR/RXR dimer.